Assessing focal liver lesions in Beckwith-Wiedemann Syndrome: Comparison of contrast-enhanced ultrasound and magnetic resonance imaging.
In a retrospective cohort of 12 infants with molecularly confirmed Beckwith-Wiedemann syndrome and focal liver lesions, CEUS showed lower reported sensitivity and specificity than MRI but better inter-rater reliability and avoided sedation in all examinations.
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In a retrospective cohort of 12 infants with molecularly confirmed Beckwith-Wiedemann syndrome and focal liver lesions, CEUS showed lower reported sensitivity and specificity than MRI but better inter-rater reliability and avoided sedation in all examinations.
Research significance
The evidence shows that CEUS can characterize liver lesions without sedation in this small cohort; it supports the inference—requiring prospective validation—that CEUS could serve as an initial triage test, reserving MRI for indeterminate or suspicious lesions and thereby reducing sedation exposure without materially compromising tumor detection.
Source abstract
PURPOSE: Beckwith-Wiedemann Syndrome (BWS) predisposes children to hepatoblastoma and other cancers and requires tumor screening with abdominal ultrasound and magnetic resonance imaging (MRI) when lesions are seen. Contrast-enhanced ultrasound (CEUS) may offer an alternative to MRI, which often requires sedation. Our aim was to evaluate the diagnostic performance of CEUS compared to MRI in this group when evaluating liver lesions, to compare sedation requirements, and to assess imaging features correlated with malignancy. MATERIALS AND METHODS: This was a retrospective review of patients with molecular BWS and liver lesions who underwent MRI and CEUS within 6 months. Demographics, sedation requirement, and final diagnosis (pathology or follow-up imaging) were recorded. Two blinded radiologists per category classified lesions (benign/malignant/indeterminate) and detailed imaging features. Test performance and inter-rater reliability (Cohen's κ) were calculated. RESULTS: 12 patients (7 females, mean age 3 months) were included. A final diagnosis of 3 hepatoblastomas and 9 benign lesions was derived from pathology in 4 cases and from imaging follow-up in 8. Ten MRI examinations (83%) and no CEUS examinations required sedation. On consensus read, CEUS had a sensitivity of 67% and a specificity of 89%; κ = 0.70. MRI had a sensitivity of 75% and a specificity of 100%; κ = 0.25. Iso-enhancement and washout on CEUS and cystic change, necrosis, and diffusion restriction on MRI correlated with malignancy. CONCLUSION: Our findings suggest that CEUS has good diagnostic accuracy and high inter-rater reliability and allows sedation avoidance, suggesting that its use could allow MRI to be reserved for indeterminate or malignant lesions.